3ojy
From Proteopedia
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| - | [[Image:3ojy.png|left|200px]] | ||
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{{STRUCTURE_3ojy| PDB=3ojy | SCENE= }} | {{STRUCTURE_3ojy| PDB=3ojy | SCENE= }} | ||
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===Crystal Structure of Human Complement Component C8=== | ===Crystal Structure of Human Complement Component C8=== | ||
| + | {{ABSTRACT_PUBMED_21454577}} | ||
| + | ==Disease== | ||
| + | [[http://www.uniprot.org/uniprot/CO8A_HUMAN CO8A_HUMAN]] Defects in C8A are a cause of complement component 8 deficiency type 1 (C8D1) [MIM:[http://omim.org/entry/613790 613790]]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. | ||
| - | + | ==Function== | |
| - | + | [[http://www.uniprot.org/uniprot/CO8A_HUMAN CO8A_HUMAN]] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C8A inserts into the target membrane, but does not form pores by itself.<ref>PMID:7440581</ref><ref>PMID:17872444</ref> [[http://www.uniprot.org/uniprot/CO8G_HUMAN CO8G_HUMAN]] C8 is a constituent of the membrane attack complex. C8 binds to the C5B-7 complex, forming the C5B-8 complex. C5-B8 binds C9 and acts as a catalyst in the polymerization of C9. The gamma subunit seems to be able to bind retinol. | |
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==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:021454577</ref><references group="xtra"/> | + | <ref group="xtra">PMID:021454577</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Cooper, C L.]] | [[Category: Cooper, C L.]] | ||
Revision as of 05:59, 25 March 2013
Contents |
Crystal Structure of Human Complement Component C8
Template:ABSTRACT PUBMED 21454577
Disease
[CO8A_HUMAN] Defects in C8A are a cause of complement component 8 deficiency type 1 (C8D1) [MIM:613790]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
Function
[CO8A_HUMAN] Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C8A inserts into the target membrane, but does not form pores by itself.[1][2] [CO8G_HUMAN] C8 is a constituent of the membrane attack complex. C8 binds to the C5B-7 complex, forming the C5B-8 complex. C5-B8 binds C9 and acts as a catalyst in the polymerization of C9. The gamma subunit seems to be able to bind retinol.
About this Structure
3ojy is a 3 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Lovelace LL, Cooper CL, Sodetz JM, Lebioda L. Structure of human C8 protein provides mechanistic insight into membrane pore formation by complement. J Biol Chem. 2011 May 20;286(20):17585-92. Epub 2011 Mar 25. PMID:21454577 doi:10.1074/jbc.M111.219766
- ↑ Steckel EW, York RG, Monahan JB, Sodetz JM. The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure. J Biol Chem. 1980 Dec 25;255(24):11997-2005. PMID:7440581
- ↑ Hadders MA, Beringer DX, Gros P. Structure of C8alpha-MACPF reveals mechanism of membrane attack in complement immune defense. Science. 2007 Sep 14;317(5844):1552-4. PMID:17872444 doi:317/5844/1552
